Bruton's agammaglobulinemia X linked

• Facts:
  X linked recessive disorder
  B cell deficiency results in inability to produce Ig
• History / PE:
  Infections begin after 6 months (when maternal ABs disappear)
  Absent tonsils
• Diagnosis:
  B cells low/absent (low CD19+ cells)
  Normal level of T cells (CD 3+ cells)
• Treatment:
  Regular IVIG infusion
• Associated With:
  Recurrent lung and sinus infections with Streptococcus and Haemophilus spp.
• Differential Diagnosis:
  Common variable immunodeficiency (acquired hypogammaglobulinemia)